Pectus excavatum y carinatum en el síndrome de Marfan y síndromes similares: prevalencia e impacto clínico pulmonar y cardiovascular
| Autor: | Leonel Avendaño, Carlos Vargas García, Nilda Espínola, C Sergio Criales, María Elena Soto, Roberto Cano |
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| Rok vydání: | 2018 |
| Předmět: |
Adult
Lung Diseases Male Marfan syndrome medicine.medical_specialty Social adjustment Adolescent Marfan Syndrome Cardiovascular symptoms Young Adult Pectus excavatum Pectus Carinatum Prevalence medicine Humans Respiratory function In patient Prospective Studies Child Gynecology business.industry General Medicine Middle Aged medicine.disease Magnetic Resonance Imaging Respiratory Function Tests Cardiovascular Diseases Echocardiography Child Preschool Funnel Chest Pectus carinatum Female Haller index Tomography X-Ray Computed business |
| Zdroj: | Gaceta Médica de México. 154 |
| ISSN: | 0016-3813 |
| DOI: | 10.24875/gmm.18004581 |
| Popis: | espanolIntroduccion: El pectus excavatum (PE) y el pectus carinatum (PC) son frecuentes en el sindrome de Marfan (SM) y en sindromes similares (SS). Los pacientes pueden evolucionar sin sintomas. En algunos hay depresion, trastornos de adaptacion social, sintomas pulmonares y cardiovasculares, en los cuales hay controversia de su relacion con el dano estructural del torax. Objetivo: Evaluar la prevalencia del tipo de deformidad toracica en pacientes con SM y SS en una cohorte historica y analizar el impacto clinico, pulmonar y cardiovascular. Metodo: Estudio prospectivo. Se incluyeron sujetos con criterios de Ghent y caracteristicas especificas de cada sindrome, con expediente completo, ecocardiograma o resonancia magnetica y tomografia computada, y pruebas de funcion respiratoria. Resultados: De un total de 338 pacientes con SM y SS, se detec- taron 112 casos con deformidad toracica. Prevalencia de PE y PC en SM: 13.6 y 12.4; fue menor en SS. Hay compresion y desplazamiento de pulmon y cavidades cardiacas derechas por PE. Hay correlacion entre el Indice de Haller y la presion sistolica de la arteria pulmonar incrementada es de 44 (p = 0.009). Conclusiones: La prevalencia de PE y PC en el SM y SS es alta, lo cual impacta en la funcion pulmonar y cardiovascular, en esas condiciones se requiere del manejo correctivo de la deformidad toracica y el objetivo no es estetico. EnglishIntroduction: Pectus excavatum (PE) and carinatum (PC) are common in Marfan syndrome (SM) and similar syndromes (SS). Patients can evolve without symptoms. In some there is depression, social adjustment disorders, pulmonary and cardiovascular symptoms in which there is controversy about their relationship with the structural damage of the thorax. Objective: To assess the prevalence of the type of thoracic deformity in patients with MS and SS in a historical and current cohort and to analyze the clinical, pulmonary and cardiovascular impact. Method: Prospective study. Subjects who met the Ghent criteria and who had a complete clinical record, an echocardiogram and/or magnetic resonance imaging, computed tomography and respiratory function tests were included. Results: Of a total of 338 patients with MS and SS, 112 cases with thoracic deformity were detected, the prevalence of PE and PC in SM 13.6 and 12.4, respectively, was lower in SS. There is compression and displacement of lung and right cardiac cavities by PE and the correlation between the Haller Index and the increased PASP is 44 (p = 0.009). Conclusions: The prevalence of PE and PC in SM and SS is high, which impacts on lung function and cardiovascular dama- ge, requires corrective management of the thoracic deformity and not only implies for aesthetic purposes. |
| Databáze: | OpenAIRE |
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